A case of reversible cerebral vasoconstriction syndrome associated with anti-phospholipid antibody syndrome and systemic lupus erythematosus

• Published in: eNeurologicalSci Vol. 24; p. 100351
• Main Authors: Shima, Atsushi, Maki, Takakuni, Mimura, Naoya, Yamashita, Hirofumi, Emoto, Noriaki, Yoshifuji, Hajime, Takahashi, Ryosuke
• Format: Journal Article
• Language: English
• Published: Elsevier B.V 01.09.2021; Elsevier
• Subjects: Anti-phospholipid antibody syndrome; Case Report; Cilostazol; Endothelin-1; Lomerizine; Reversible cerebral vasoconstriction syndrome; Lomerizine; Cilostazol; Reversible cerebral vasoconstriction syndrome; Anti-phospholipid antibody syndrome; Endothelin-1
• ISSN: 2405-6502; 2405-6502
• DOI: 10.1016/j.ensci.2021.100351

The pathomechanisms and treatment strategy for rare presentations of reversible cerebral vasoconstriction syndrome (RCVS) with anti-phospholipid syndrome (APS) remain to be determined. We report a 67-year-old woman with APS who presented with ischemic stroke due to RCVS. She was treated with low-dose cilostazol and lomerizine hydrochloride, which resulted in functional improvement and recovery of vasoconstriction within 12 weeks. Her plasma endothelin-1 level was decreased after relief of vasoconstriction, compared with the pre-treatment condition. Increased plasma endothelin-1 may be related to the underlying pathomechanism of RCVS with APS, against which cilostazol and lomerizine hydrochloride could be effective. •We described a case of reversible cerebral vasoconstriction syndrome (RCVS) with anti-phospholipid syndrome (APS).•Treatment with low-dose cilostazol and lomerizine hydrochloride could therapeutic for RCVS with APS.•These treatments may alleviate the endothelin-1-related cerebral vascular dysfunction in RCVS/APS.